Congenital cysts and fistulas of the neck

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Abstract

This retrospective study describes a series of 191 children treated for congenital cysts and fistulas of the neck between 1984 and 1999 in the pediatric ORL Department of La Timone Children's Hospital. Preauricular fistulas and cystic hygromas were not included. The anomalies in this series were classified as either malformations of the midline or malformations of laterocervical region. Malformations of the midline included the thyroglossal duct cysts (n=102) and dermoid cysts (n=21). The most common malformations of the laterocervical region were cysts and fistulas of the second cleft (n=37) followed by those of the first cleft (n=20),those of the fourth pouch (n=7), and thymic cysts (n=4). Diagnosis of malformations of the midline is usually straightforward. However, diagnosis of malformation of the laterocervical region can be problematic. Misdiagnosis often leads to inadequate treatment with recurrence and functional as well as cosmetic sequelae.

Introduction

Some congenital anomalies of the neck are commonplace in pediatric practice. This is the case for thyroglossal tract cysts, unilocular branchial cysts, and fistulas of the second cleft. However, less common lesions such as fistulas of the first cleft and cysts of the fourth pouch may pose problems of misdiagnosis and recurrence. Malformation of the midline such as thyroglossal duct cysts and dermoid cysts are embryologically different from malformation of the laterocervical region. Awareness of the characteristic anatomic and clinical features of each lesion is essential not only to allow accurate diagnosis but also to achieve complete surgical excision for recurrence-free treatment. The purpose of this report is to present our 15-year experience in the management of congenital anomalies of the neck in children. Cystic hygromas and preauricular fistulas were not included in this study.

Section snippets

Embryological background

Embryological development of the laterocervical region is closely linked with that of the branchial apparatus which forms within the first month of intrauterine life. In man, the branchial arches are arranged in pairs on either side of the midline. Morphologically, the apparatus can be described as a succession of folds and grooves corresponding to the branchial clefts and pouches. Four arches can be clearly identified on the embryo. A rudimentary fifth arch appears but quickly regresses [1].

Patients and methods

Between 1984 and 1999, 191 children underwent surgical treatment for congenital cysts or fistulas of the neck in the pediatric department of Otolaryngology of La Timone Children's Hospital in Marseille, France. Embryologically, the anomalies in this series were classified as either malformations of the midline or malformation of the laterocervical region (Table 2). Anatomically the lesions could be classified as incomplete fistulas presenting a dead-end fistulous tract, complete fistulas

Laterocervical malformations

In patients involving malformations of the first arch, the course of the fistulous tract passed above the facial nerve in 12 patients, below the facial nerve in five, and between the branches of the facial nerve in two. Recurrence was observed during postoperative recovery in two children (10%) requiring re-operation within nine months. One newborn who demonstrated facial paralysis in relation with a suppurative infection of the lesion achieved complete recovery after surgery. With a mean

Discussion

Study of the cervical region has shown that the midline and laterocervical regions are embryologically distinct. Development of the laterocervical regions depends on the branchial arches while that of the midline depends on proper closure of the embryo. This difference explains the distinction that must be made between malformations of the laterocervical region and malformations of the midline.

Most reports in the literature have used the system proposed by Work (in [3]) for classification of

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